NEUROLOGICAL AND DEVELOPMENTAL OUTCOME IN TOF

这个子项目是许多研究子项目中的一个 由NIH/NCRR资助的中心赠款提供的资源。子项目和 研究者（PI）可能从另一个NIH来源获得了主要资金， 因此可以在其他CRISP条目中表示。所列机构为 研究中心，而研究中心不一定是研究者所在的机构。 AIM 1. 比较TOF组与对照组在学习成绩、认知和行为方面的差异。 假设：与正常对照组相比，不具有22 q11微缺失的TOF修复儿童将表现出学业，认知和行为缺陷。 主要结果将是韦氏个人成就测试（WIAT）的总综合评分。 WIAT评估的学业成功代表了包括智力、神经心理学优势和弱点以及心理健康在内的因素的结果。 因此，次要结局将包括以下内容：（1）能力，采用韦氏儿童智力量表（第三版）的全量表、言语和操作智商进行测量（2）学习障碍，定义为儿童在特定学术领域的能力和成就之间的显著差异;（3）在特定神经心理学领域的表现，包括语言、记忆、注意力、执行功能和视觉空间/运动整合;和（4）精神健康，包括生活质量和整体情绪功能，以及特定的精神病诊断，包括情绪、焦虑、注意力缺陷/破坏性行为和精神障碍。 AIM 2.比较TOF组和对照组的脑结构测量结果。 假设：TOF修复的儿童不具有22 q11缺失，其结构MRI结果与匹配的对照组不同。 使用定量体积MRI成像和分割的主要结局将是总脑组织体积。 次要结局将是灰质（总灰质、皮质灰质和皮质下灰质）、白色物质（有髓和无髓白色物质）和脑脊液的体积。 使用弥散张量MRI成像的主要结果变量将是通过相对各向异性（RA）测量的局部白色物质微结构。

This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. AIM 1. To compare TOF and control groups on academic achievement, cognition and behavior. Hypothesis: Children with repaired TOF who do not possess 22q11 microdeletion will demonstrate academic, cognitive, and behavioral deficiencies relative to normal matched controls. The primary outcome will be Total Composite Score on the Wechsler Individual Achievement Test (WIAT). Academic success, as assessed by the WIAT, represents the outcome of factors including intelligence, neuropsychological strengths and weaknesses, and mental health. Therefore, secondary outcomes will include the following: (1) ability, measured by the Full-Scale, Verbal, and Performance IQ on the Wechsler Intelligence Scale for Children-Third Edition (WISC-III); (2) learning disability, defined as a significant discrepancy between a child's ability and achievement within particular academic domains; (3) performance in specific neuropsychologic domains, including language, memory, attention, executive functions, and visual-spatial/motor integration; and (4) mental health, including quality-of-life and global emotional functioning, as well as specific psychiatric diagnoses including mood, anxiety, attention-deficit/disruptive behavior, and psychotic disorders. AIM 2. To compare TOF and control groups on measures of brain structure. Hypothesis: Children with repaired TOF who do not possess the 22q11 deletion will have findings on structural MRI that differ from matched controls. The primary outcome using quantitative volumetric MRI imaging and segmentation will be total brain tissue volume. Secondary outcomes will be volumes of gray matter (total, cortical, and subcortical gray matter), white matter (myelinated and unmyelinated white matter), and cerebrospinal fluid. The primary outcome variable using diffusion tensor MRI imaging will be regional white matter microstructure, as measured by relative anisotropy (RA).